Guillain-Barré Syndrome

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

PMP22, CD86, AIRE, TNF, IL17A, CSF2, LAMC2, CD1E, ALB, CD1A, HLA-DQB1, IL1B, CD1C, TLR4, IL10, CD1B, ICAM1, IL23A, ISG20, FCGR2A, CRP, IL4, IL2RA, IL17D, HLA-DRB1, IL6, MAPK1, NFASC, GFAP, ITGAM

PMP22, CD86, AIRE, TNF, IL17A, CSF2, LAMC2, CD1E, ALB, CD1A, HLA-DQB1, IL1B, CD1C, TLR4, IL10, CD1B, ICAM1, IL23A, ISG20, FCGR2A, CRP, IL4, IL2RA, IL17D, HLA-DRB1, IL6, MAPK1, NFASC, GFAP, ITGAM, COX2, MYDGF, PTGS2, FCGR3A, APOE, IL27, FAS, MTCO2P12, CNTNAP1, YY1, YWHAZ, CXCR4, AIMP2, RETN, SELENBP1, TNFRSF1B, MIR155, GRAP2, GLDN, NOD1, VIM, MIR642B, SIGLEC14, AHSA1, OCLN, TLR2, TH, TGFB1, STAT3, SPP1, SMPD2, CXCL6, CCL2, PTPN11, MIF-AS1, IVNS1ABP, CSGALNACT1, POLDIP2, SMPD3, NS2, IL21, ISYNA1, FOXP3, IL22, ICOS, DLL1, ERVK-6, SIGLEC9, ST6GALNAC4, PTGS1, RNF19A, TUBGCP2, NOD2, SUMF2, FTSJ1, IL33, CADM1, FCRL3, FTSJ3, RBM45, HT, CABIN1, PLB1, YWHAQ, SIGLEC7, NOS2, PSMB6, F2R, HMGB1, HLA-DQB2, HLA-DQA1, HLA-DOA, CXCL2, CXCL1, NR3C1, GOLGA4, GNAO1, GLO1, GJB1, GALE, FOLH1, FCGR3B, ESR1, HPRT1, EPHB2, ENO2, CST3, MAPK14, CRK, COX8A, CNTN1, CDC42, CD59, CD80, CD14, CACNA1A, SERPING1, FASLG, HP, HSPA4, MAP2K7, MMP2, MAPK8, PRKD1, POMC, PLA2G1B, PKD1, PGF, PDCD1, NPPB, NPY, NOS3, NEFL, COX1, MRC1, MMP9, NR3C2, HSPD1, MIF, MBL2, LEP, ITGB2, ITGAL, IRF6, INSRR, CXCL10, IL18, IL12B, IL12A, IL2, IGHG3, IFNG, ERVK-32

Drugs

4-aminopyridine, Coversin, Fampridine ( AMPYRA )

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A clinically heterogeneous spectrum of rare post-infectious neuropathies that usually occur in otherwise healthy patients and encompasses acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS) and some other regional variants.

Epidemiology

The overall annual incidence of GBS varies between 1/91,000 and 1/55,000. In Europe and North America, AIDP is the most frequent form of GBS (accounting for around 90% of cases) and thus the term GBS in general is synonymous with AIDP in Western countries. The axonal forms account for only 3-5% of cases in Western countries but are much more frequent (30%-50% of GBS cases) in Asia and Latin America.

Etiology

In the majority of cases, an infectious disease precedes the onset of limb weakness with Campylobacter jejuni infection being the most frequently identified initiating event. GBS has also been reported to occur after vaccination or following a surgical intervention.

Management and treatment

Treatment consists of rapid administration of intravenous immunoglobulin (IVIg) or plasma exchange (PE). Physiotherapy and rehabilitation are also important.

Prognosis

The prognosis is variable depending on the form of GBS and ranges from patients with complete recovery, to those who are unable to walk 6 months after disease onset, and to patients in whom the disease has a fatal outcome.